Introduction Defects in the maturation stage of amelogenesis result in a normal volume of enamel but insufficient mineralization, called hypomineralization. Molar‐incisor hypomineralization (MIH), amelogenesis imperfecta and dental fluorosis (DF) are examples of such defects. Objective To evaluate the effectiveness of the treatments applied to the different forms of dental hypomineralization. Materials and Methods PubMed, Scopus, Cochrane Library, Web of Science, and Embase were screened. The research was limited to studies published in English, Spanish, and Portuguese, until May 30, 2018. The research question was formulated following the Population, Intervention, Comparison, Outcome strategy. The quality of the methodology of each article was evaluated employing the Cochrane Handbook for Systematic Reviews. Results From the initial research, 7895 references were obtained, of which 33 were included in the systematic review. The following treatments were reported: desensitizing and remineralizing products, resin infiltration, restorations, fissure sealants, tooth bleaching, enamel microabrasion and calcium, and vitamins supplements. Conclusions Although the results are suggestive, there is a clear need for a greater uniformity of the methodologies, thus allowing for the development of clinical guidelines. Nevertheless, it was possible to identify several effective treatments for teeth with MIH (arginine pastes or fluoride varnishes) and DF (tooth bleaching and/or enamel microabrasion). Clinical Significance Because MIH, amelogenesis imperfecta, and DF are commonly seen in dental daily practice, it is extremely important to analyze the literature regarding its treatment.